scholarly journals 10-year comparative follow up of familial versus multifactorial chylomicronemia syndromes

Author(s):  
Manon Belhassen ◽  
Eric Van Ganse ◽  
Maeva Nolin ◽  
Marjorie Bérard ◽  
Hanane Bada ◽  
...  

Abstract Context The relative incidence of acute pancreatitis, ischemic cardiovascular disease and diabetes in hyperchylomicronemic patients exhibiting familial chylomicronemia syndrome (FCS), or multifactorial chylomicronemia syndrome (MCS), is unknown. Objective The objective was to study the occurrence of these events in FCS and MCS patients compared with the general population. Methods Twenty-nine FCS and 124 MCS patients, with genetic diagnosis, in four lipid clinics were matched with 413 controls. Individual hospital data linked to the national claims database were collected between 2006 and 2016. The occurrence of complications was retrospectively assessed before follow-up and during a median follow-up time of 9.8 years, for 1500 patient years of follow-up. Results FCS were younger than MCS (34.3 ± 13.6 vs 45.2 ± 12.6 years, p<0.01). During the study period, 58.6% of the FCS patients versus 19.4% of the MCS patients had at least one episode of acute hypertriglyceridemic pancreatitis (AHP) (HR=3.6; p<0.01). Conversely, the ischemic risk was lower in FCS than in MCS (HR=0,3; p=0.05). The risk of venous thrombosis was similar in both groups. The incidence of diabetes was high in both groups compared with matched controls (OR=22.8; p<0.01 in FCS and OR=30.3; p<0.01 in MCS). Conclusion The incidence of AHP was much higher in FCS than in MCS patients, whereas the incidence of ischemic cardiovascular events was found increased in MCS both versus FCS and a representative matched control group. Differences in both triglycerides-rich lipoprotein metabolism and comorbidities in MCS vs FCS drive the occurrence of different patterns of complications.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Anna-Lotta Irewall ◽  
Anders Ulvenstam ◽  
Anna Graipe ◽  
Joachim Ögren ◽  
Thomas Mooe

AbstractEnhanced follow-up is needed to improve the results of secondary preventive care in patients with established cardiovascular disease. We examined the effect of long-term, nurse-based, secondary preventive follow-up by telephone on the recurrence of cardiovascular events. Open, randomised, controlled trial with two parallel groups. Between 1 January 2010 and 31 December 2014, consecutive patients (n = 1890) admitted to hospital due to stroke, transient ischaemic attack (TIA), or acute coronary syndrome (ACS) were included. Participants were randomised (1:1) to nurse-based telephone follow-up (intervention, n = 944) or usual care (control, n = 946) and followed until 31 December 2017. The primary endpoint was a composite of stroke, myocardial infarction, cardiac revascularisation, and cardiovascular death. The individual components of the primary endpoint, TIA, and all-cause mortality were analysed as secondary endpoints. The assessment of outcome events was blinded to study group assignment. After a mean follow-up of 4.5 years, 22.7% (n = 214) of patients in the intervention group and 27.1% (n = 256) in the control group reached the primary composite endpoint (HR 0.81, 95% CI 0.68–0.97; ARR 4.4%, 95% CI 0.5–8.3). Secondary endpoints did not differ significantly between groups. Nurse-based secondary preventive follow-up by telephone reduced the recurrence of cardiovascular events during long-term follow-up.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 100.3-100
Author(s):  
Y. Wang ◽  
X. Liu ◽  
Y. Shi ◽  
X. Ji ◽  
W. Wang ◽  
...  

Background:Clinical practice guidelines recommend that exercise is an essential component in the self-management of Ankylosing Spondylitis (AS). Attending supervised interventions requiring periodic medical center visits can be burdensome and patients may decline participation, whereas, effective home-based exercise interventions that do not need regular medical center visits are likely to be more accessible and acceptable for patients with AS. Recently, increasing evidences have been accumulated that the wearable devices could facilitate patients with inflammatory arthritis by giving exercise instructions and improving self-efficacy. Therefore, patients with AS may benefit from an effective technology-assisted home-based exercise intervention.Objectives:To investigate the efficacy of a comprehensive technology-assisted home-based exercise intervention on disease activity in patients with AS.Methods:This study was a 16-week assessor-blinded, randomized, waiting-list controlled trial (ChiCTR1900024244). Patients with AS were randomly allocated to the home-based exercise intervention group and the waiting-list control group. A 16-week comprehensive exercise program consisting of a moderate intensity (64%-76% HRmax) aerobic training for 30min on 5 days/week and a functional training for 60min on 3 days/week was given to patients in the intervention group immediately after randomization, with 1.5h training sessions for two consecutive days by a study physical therapist at baseline and Week 8. The aerobic exercise intensity was controlled by a Mio FUSE Wristband with a smartphone application. The functional training consisted of the posture training, range of motion exercises, strength training, stability training and stretching exercises. Patients in control group received standard care during the 16-week follow-up and started to receive the exercise program at Week 16. The primary outcome was ASDAS at Week 16. The secondary outcomes were BASDAI, BASFI, BASMI, ASAS HI, peak oxygen uptake, body composition and muscle endurance tests. The mean difference between groups in change from baseline was analyzed with the analysis of covariance.Results:A total of 54 patients with AS were enrolled (26 in intervention group and 28 in control group) and 46 (85.2%) patients completed the 16-week follow-up. The mean difference of ASDAS between groups in change from baseline to 16-week follow-up was −0.2 (95% CI, −0.4 to 0.003, P = 0.032), and the mean change from baseline was -0.4 (95% CI, -0.5 to -0.2) in the intervention group vs -0.1 (95% CI, -0.3 to 0.01) in the control group, respectively. Significant between-group differences were found between groups for BASDAI (−0.5 [95% CI, −0.9 to −0.2], P = 0.004), BASMI (−0.7 [95% CI, −1.1 to −0.4], P <0.001), BASFI (−0.3 [95% CI, −0.6 to 0.01], P=0.035), peak oxygen uptake (2.7 [95% CI, 0.02 to 5.3] ml/kg/min, P=0.048) and extensor endurance test (17.8 [95% CI, 0.5 to 35.2]s, P=0.044) at Week 16. Between-group differences were detected in ASAS HI (−0.9 [95% CI, −1.7 to −0.1], P=0.030), body fat percentage (−1.0 [95% CI, −2.0 to −0.01] %, P=0.048) and visceral adipose tissue (−4.9 [95% CI, −8.5 to −1.4] cm2, P=0.008) at Week 8, but not at Week 16. No significant between-group differences were detected in the total lean mass, time up and go test and the flexor endurance test during the follow-up.Conclusion:Comprehensive technology-assisted home-based exercise has been shown to have beneficial effects on disease activity, physical function, spinal mobility, aerobic capacity, and body composition as well as in improving fatigue and morning stiffness of patients with AS.References:[1]van der Heijde D, Ramiro S, Landewé R, et al. Ann Rheum Dis 2017;76:978–991.Disclosure of Interests:None declared


2020 ◽  
Vol 49 (1) ◽  
pp. 66-75 ◽  
Author(s):  
David R. Maldonado ◽  
Cynthia Kyin ◽  
Jacob Shapira ◽  
Philip J. Rosinsky ◽  
Mitchell B. Meghpara ◽  
...  

Background: Hip arthroscopy in patients with borderline dysplasia continues to be surrounded by controversy. Even more controversial is the management of the failed hip arthroscopy in this population. There is a paucity of studies in contemporary literature regarding outcomes after arthroscopic revision surgery. Purpose: (1) To report minimum 2-year patient-reported outcome (PRO) scores in patients with borderline dysplasia who underwent revision hip arthroscopy and (2) to compare these PRO scores with those of a propensity-matched control group without dysplasia who underwent revision hip arthroscopy. Study Design: Cohort study; Level of evidence, 3. Methods: Data were prospectively collected between August 2009 and November 2017. Inclusion criteria were revision arthroscopic surgery, capsular plication, and baseline and minimum 2-year follow-up for the following PROs: modified Harris Hip Score (mHHS), Nonarthritic Hip Score (NAHS), Hip Outcome Score–Sports Specific Subscale (HOS-SSS), and visual analog scale (VAS) for pain. Patients with Tönnis grade >1 or previous hip conditions were excluded. Two groups were created: a study group with borderline dysplasia (lateral center-edge angle [LCEA], 18°-25°) and a control group without dysplasia (LCEA, 25°-40°). Groups were propensity-matched in a 1:3 ratio for sex, age, body mass index, and follow-up time. Results: A total of 22 revision borderline dysplastic hips (21 patients) had a minimum 2-year follow-up during the study period. Patients in this group reported significant improvements for all PROs from baseline and achieved the minimal clinically important difference (MCID) for the mHHS at a rate of 70%. Moreover, 21 borderline dysplastic hips (21 patients) were matched to 63 control hips (63 patients). Mean LCEA for the study and control groups was 22.6 ± 1.7 and 32.0 ± 5.0, respectively. Both groups reported similar improvement in all PROs. The rate for achieving the MCID for the mHHS and VAS was similar between groups; however, the control group had higher rates of meeting the MCID for the HOS-SSS and NAHS ( P = .042 and P = .025, respectively). The rates of conversion to hip arthroplasty were 7.9% (n = 5) in the control group and 23.8% (n = 5) in the borderline dysplasia propensity-matched group ( P = .052). The rate of re-revision arthroscopy was 11.1% (n = 7) in the control group and 19.0% (n = 4) on the borderline dysplasia group ( P = .350). Conclusion: After revision hip arthroscopy, significant improvement was obtained for all PROs in patients with borderline dysplasia at a minimum 2-year follow-up. Moreover, outcomes, patient satisfaction, the rate for achieving the MCID for the mHHS and VAS, and the rate for secondary surgery were similar to those of a propensity-matched control group without dysplasia. Nevertheless, there was a nonsignificant trend toward higher secondary procedures in the study group; therefore, arthroscopic revision surgery in the borderline patients should be approached with measured prognosis.


1980 ◽  
Vol 10 (1) ◽  
pp. 85-100 ◽  
Author(s):  
Sula Wolff ◽  
Jonathan Chick

SyonpsisTwenty-two boys with schizoid personality were followed-up some 10 years later and compared with a matched control group with other diagnoses who had been referred to the same child psychiatry department. The diagnostic category is shown to have predictive validity. A start has been made towards an operational definition of the syndrome.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
V Rezapova ◽  
D Usoltsev ◽  
O Rotar ◽  
R Libis ◽  
D Duplyakov ◽  
...  

Abstract Introduction Cardiovascular diseases (CVD) are the most serious public health problem, remaining the leading cause of death in the adult population. A great number of CVD risk scales are successfully used in clinics, yet they are tuned to work the best for patients of older age and often are population specific. Methods We used the data from a longitudinal epidemiologic study of 4,750 individuals aged 25–64 years from European part of Russia recruited in 2012–2013 to predict the 10-year risk of cardiovascular events in Russian population and evaluate performance for common clinical risk scales. The cohort was divided into two groups: “cases” – individuals with a cardiovascular event registered during the follow up period (2013–2019) as a study group (N=106) and “controls” – individuals with no cardiovascular events registered during follow up as a control group (N=4,644). All individuals with previous history of CVD (ischemic heart disease, stroke or heart attack) prior to 2012 were assigned high risk in all scales. The case and control groups were split by age: below and above 40 to reflect the properties of cardiovascular risk scales to work best for age over 40. We assessed CVD risk scores using Framingham 2008 [1], ASCVD 2013, both were calculated with mean parameters values calculated from our cohort and from the original study [2], SCORE 2003, 2017 and 2019 [3] and MOSP - the recalibrated for Russian population scale of the SCORE 2017 [4] Results For patients over 40 years old, cardiovascular risk scales showed similar performance, with ASCVD normalized to the original study's mean parameter values returning the best prediction scores. Expectedly, for the younger group of patients, cardiac risk scales do not have notable predictive power. Further, we thought to identify additional factors discriminating young individuals at higher risk of CVD. We downsampled the control cohort to include only samples with age, weight, height, LDL, total cholesterol and systolic blood pressure matching those in a case cohort (&lt;40 years of age). 186 phenotypic features were tested and 4 out of top 10 (p&lt;0.05) associated features included questions related to depression, anxiety or current stress level from HADS scale, though not significant after multiple hypothesis correction, this suggests that younger individuals with high scores in depression/anxiety screenings might be at higher risk of CVD. Depression/anxiety features were added to cardiovascular risk scales as binary predictor components to improve prediction quality (Table 1). Conclusions We identified depression and anxiety screening questionnaires as a valuable predictor for cardiovascular events in the younger population improving the quality of predictions of traditional clinical scales. FUNDunding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Ministry of Science and Higher Education of the Russian Federation Table 1. ROC analysis


2021 ◽  
Vol 34 (4) ◽  
pp. e100452
Author(s):  
Alison Ann Tebbett-Mock ◽  
Madeline McGee ◽  
Ema Saito

BackgroundDialectical behaviour therapy (DBT) is an evidence-based treatment for adolescents targeting suicidal and non-suicidal self-injurious behaviours. Research supports DBT’s efficacy in inpatient settings, but implementation and sustainability are understudied.AimsThis study is a follow-up of a previous study by Tebbett-Mock et al and examines the efficacy and sustainability of an adolescent DBT inpatient unit within a psychiatric hospital in the Northeast. We hypothesised that adolescents who received DBT in our follow-up group (DBT Group 2) would not have statistical difference (ie, greater or fewer) of the following compared with the first group of patients who received DBT on the unit the year prior (DBT Group 1) and would have significantly fewer of the following compared with the treatment as usual (TAU) group: (1) constant observation hours for suicidal ideation, self-injury and aggression; (2) incidents of suicide attempts, self-injury and aggression; (3) restraints; (4) seclusions; (5) days hospitalised; (6) times readmitted to the unit within 30 days of discharge.MethodsWe conducted a retrospective chart review for adolescents receiving inpatient DBT (DBT Group 1, n=425; DBT Group 2, n=393) and a historical control group (TAU, n=376). The χ2 tests and one-way analysis of variance were conducted as preliminary analyses to examine group differences on diagnosis, gender and age. Kruskal-Wallis H tests were conducted to examine group differences on outcomes. Mann-Whitney U tests were used as post hoc analyses.ResultsPatients in DBT Group 2 were comparable to DBT Group 1 for the number of constant observation hours for self-injury (U=83 432.50, p=0.901), restraints (U=82 109, p=0.171) and days hospitalised (U=83 438.5, p=0.956). Patients in DBT Group 2 had a significantly greater number of incidents of suicide attempts compared with DBT Group 1 (U=82 662.5, p=0.037) and of self-injury compared with patients in DBT Group 1 (U=71724.5, p<0.001) and TAU (U=65649.0, p<0.001).ConclusionsResults provide support for adolescent inpatient DBT compared with TAU and highlight staff turnover and lack of training as potential barriers to sustainability and efficacy.


CNS Spectrums ◽  
2019 ◽  
Vol 24 (04) ◽  
pp. 453-459 ◽  
Author(s):  
Mao-Hsuan Huang ◽  
Mu-Hong Chen ◽  
Kai-Lin Huang ◽  
Ju-Wei Hsu ◽  
Ya-Mei Bai ◽  
...  

BackgroundResearch suggests an association between metabolic disorders, such as type 2 diabetes mellitus (T2DM), and schizophrenia. However, the risk of metabolic disorders in the unaffected siblings of patients with schizophrenia remains unclear.MethodsUsing the Taiwan National Health Insurance Research Database, 3135 unaffected siblings of schizophrenia probands and 12,540 age-/sex-matched control subjects were included and followed up to the end of 2011. Individuals who developed metabolic disorders during the follow-up period were identified.ResultsThe unaffected siblings of schizophrenia probands had a higher prevalence of T2DM (3.4% vs. 2.6%, p = 0.010) than the controls. Logistic regression analyses with the adjustment of demographic data revealed that the unaffected siblings of patients with schizophrenia were more likely to develop T2DM (odds ratio [OR]: 1.39, 95% confidence interval [CI]: 1.10–1.75) later in life compared with the control group. Moreover, only female siblings of schizophrenia probands had an increased risk of hypertension (OR: 1.47, 95% CI: 1.07–2.01) during the follow-up compared with the controls.DiscussionThe unaffected siblings, especially sisters, of schizophrenia probands had a higher prevalence of T2DM and hypertension compared with the controls. Our study revealed a familial link between schizophrenia and T2DM in a large sample. Additional studies are required to investigate the shared pathophysiology of schizophrenia and T2DM.


2020 ◽  
Vol 79 (OCE2) ◽  
Author(s):  
Leigh-Ann McCrum ◽  
Gerry Mckenna ◽  
Jayne Woodside ◽  
Laura McGowan ◽  
Sinead Watson

AbstractIntroductionReplacing missing teeth alone is not enough to engender dietary behaviour change amongst older adults. Whilst there is a body of evidence to support oral rehabilitation in conjunction with dietary advice, this is currently limited to edentate patients even though the majority of older adults are now partially dentate. One approach proven to change long-term food behaviours but is novel in this population is habit-formation. Consequently, this study developed and tested a habit-based tailored dietary intervention, in conjunction with oral rehabilitation amongst partially dentate older adults.Materials and methodsA pilot randomised control trial was conducted on 57 partially dentate older patients. Participants were randomised to an intervention group (habits-based dietary intervention) or a control group and followed up for 8 months. The intervention group attended four meetings with a trained researcher to target habit-formation around 3 dietary domains (fruit/vegetables, wholegrains, healthy proteins). The primary outcome measure was self-reported automaticity for developing healthy habits and habit formation was assessed using the Self-Report Behavioural Automaticity Index (SRBAI). Preliminary analysis was conducted on n = 36 participants between baseline and 8 month follow up.ResultsPreliminary results showed that SRBAI scores and self-reported frequency of days doing habits in the intervention group for all tailored dietary habits was significant between baseline and follow up visits (p < 0.001). There were moderate positive correlations between automaticity and habit adherence (Fruit/vegetables rho = 0.43, p = 0.09: Wholegrains rho = 0.44, p = 0.08: Healthy Proteins rho = 0.52, p = 0.03) for the intervention group. Automaticity trends were increased in the intervention group for all 3 dietary habits compared to the control group but, other than wholegrain (p = 0.005), between group differences were non-significant (p > 0.05). BMI decreased in the intervention group (29.6 to 28.7 kg/m2) compared to a non-significant increase in the control group (27.7 to 27.8 kg/m2) (p = 0.08). There were slight increases in Mini Nutritional Assessment mean change scores (0.19 intervention: 0.32 control) for both groups, however between-group differences were not statistically significant (p = 0.9). Greater improvements in food intake around dietary habits were observed in the intervention group (Fruit/vegetables:108 g Fibre 4g: Protein 11g) compared to the control group (Fruit/vegetables -17g: Fibre 2g: Protein -4g).DiscussionPreliminary results demonstrate the success of a habit-based dietary intervention coupled with oral rehabilitation in positively influencing dietary behaviours and other nutritional outcomes in partially dentate older adults.


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